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58 Cards in this Set
- Front
- Back
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ECM |
extracellular matrix |
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Osteogenesis imperfecta |
inherited disorder of collagen characterized by weak bones |
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characterized by hyperextendible joints and stretchy skin |
Ehlers-Danlos syndrome |
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Marfan syndrome- |
defective fibrillin-1 impairs the maintenance of elastin and results in defects in aorta, eye, skeleton |
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peripheral proteins function- |
*cytoskeletal proteins that attach to the membrane, regulate its shape, stabilize it
*involved in cell signaling, enzymes attached to inner membrane |
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function of lipid rafts |
cholesterol transport, endocytosis, signal transduction |
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CAVEOLAE |
cholesterol-enriched membrane invaginations (lipid raft), posses protein calveolin |
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cytosol- |
liquid portion of the cytoskeleton |
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organelles involved in expression of DNA |
nucleus ribosomes endoplasmic reticulum (ER) Golgi complex |
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every normal human cell contain... within nucleus of every cell |
23 pairs of chromosomes |
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the outer most structure of nucleus |
nuclear enveolpe |
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nuclear envelope structure |
double-layered phospholipid membrane with nuclear pores |
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nuclear envelope function |
transfer of materials between the nucleus and cytosol |
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nucleoplasm |
interior of nucleus, fluid in which chromosomes are found |
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nucleoplasm is organized by (forms associations between DNA and inner nuclear membrane) |
nuclear lamina |
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nuclear lamina |
protein composed of intermediate filaments |
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nucleus "suborganelle" |
nucleolus |
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nucleolus main function |
is the site of ribosome production ! |
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ribosomes- structure |
proteins(40%) + ribosomal RNA(60%)
small subunit (1RNA + 30 proteins) + large subunit (3RNA molecules + 50 proteins) |
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ribosomes function |
protein synthesis during translation from messenger RNA |
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where can I found ribosomes ? |
free in cytosol or bound to the RER |
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ER- endoplasmic rediculum is contiguous with... |
outer layer of nuclear envelope |
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ER in muscle cells... |
sacroplasmic reticulum |
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ER lumen might expand to... |
sac or cisternae |
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region of ER where ribosomes are bound... |
Rough endoplasmic reticulum |
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Golgi complex three regions: |
cis medial trans |
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cis |
the closest to ER |
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trans |
near to the plasma membrane |
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Golgi function |
modification of proteins: glycosylation- addition of carbohydrate, phosphorylation, proteolysis- enzyme mediated break down |
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trans network functions |
sorts, packages proteins (transport vesicles) |
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what is so special about mitochondria? |
*ATP generator *can self replicate *contain their own DNA *2 membranes *facilitate the apoptotic death |
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inner mitochondrial membrane forms folded structure called... |
cristae |
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mitochondrial lumen |
matrix |
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so how the energy is formed |
protons are pumped out of the matrix (movement of electrons, between protein complex), creating electrochemical gradient in intermembrane space, flow of protons back to the matrix drives ATP formation from proces of oxidative phosphorylation |
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Energy generated per one glucose molecule in red blood cells (lack of mitochondria) |
2 ATP |
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Energy produced from one glucose molecule in human cells with mitochondria |
32 ATP |
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mitochondria self replicate or divide by |
FISSION |
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mitochondria in programmed cell death |
proapoptotic protein insert into mitochondrial membrane ---> formation of pores---> protein Cytochrome c can leave intermembrane space to cytosol---> cascade of events resulting in cell death |
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Kearns-Sayre syndrome |
mitochondrial disorder defective mtDNA, paralysis of eye muscles, degeneration of retina |
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Pearson syndrome |
bone marrow and pancreas dysfunction, mitochondrial cytopathies |
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LYSOSOMES pH, formation |
acidic internal ph 5 formed from Golgi complex
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lysosomes enzymes |
acid hydrolases- break down proteins, nucleic acids, carbohydrates, lipids |
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acid hydrolases are synthesized ,.. |
on ribosomes bound to ER |
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Hurler syndrome |
usually stop developing between 2 and 4 years old, accumulation of glycosaminoglycans -lysosomal storage disease |
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Faber disease |
lysosomal ceramide accumulation first year of life |
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Tay Sachs disease |
accumulation of gangliosidase in brain, death by 4 |
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GAUCHER syndrome, most common lysosomal storage disease |
type I deficiency of glucosylceramidase, more lipid, splenomegaly, bone pain type II more severe, neurological (3) |
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Peroxisomes posses hydrolytic enzymes however... |
They are formed from regions of ER (as opposed to regions of the Golgi likr lysosomes) |
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Peroxisomes breake |
Fatty acids, purines AMP, GTP HYDROGEN PEROXIDE a toxic by product of many metabolic reactions
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Peroxisomes synthesis |
within hepatocytes chresterol and bile acid myelin protective sheath of neurons |
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Zewellweger syndrome |
costed by defect in transporting of peroxisomal enzymes into peroxisomes in liver, kindeys and brain (6 months) |
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All proteins are degraded by cell's .... |
PROTEOLYTIC SYSTEM |
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We can divide the proteolytic system into two different ways of degradations of proteins |
Lysosomal ATP dependent peroxysomal |
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Short life proteins s/min are degraded in cytosol using... |
ATP dependent protein degradation (proteosomes) ( |
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Membrane proteins, extracellular proteins, proteins with long half lives are degradated by... |
Lysosomal utilization |
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During cellular stress eg starving proteins are becoming source of energy by.... |
Autophagy through the lydosomal pathway |
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In chronic degenerative diseases such Huntingtons, Alzheimers, Parkinson there is... |
Abnormal accumulation of defective proteins in nervous tissue |
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Proteins destined for degradation are covalently attached to ... |
Chain of UBIQUITIN residues |
